Hello, thank you for participating in the session today, Delayed Metastasis of Wilms Tumor Occurring After More Than 40 Years. I'm Dr. Victoria Walker, Pulmonary Critical Care Fellow at Morehouse in Atlanta. I have no financial disclosures. Today I'm going to present a unique case of Wilms tumor in an adult, then we will consider some standout elements from the pathology workup and finally have a discussion. A 43-year-old woman comes to our hospital with difficulty breathing. About five months prior, though, she had been to an outside hospital for similar symptoms. Her workup there identified a large mass in the left upper lobe and a biopsy was performed. The pathology report finalized a diagnosis of neuroendocrine tumor. She had a past medical history to include hypertension, GERD, and her social history was notable for homelessness. At the time of presentation, it was not yet known to our team that the patient actually had a nephroblastoma or Wilms tumor in early childhood. The primary tumor, along with the lung metastasis, were resected when she was just three years old. CT tests with contrast revealed a large mass filling the left hemothorax, which appeared to invade the parietal pericardium. It appeared the patient at least had segmentectomies in the past and the surgical clip was engulfed by the mass and surgical absence of her kidney was also noted. After the left pneumonectomy at our facility, the resected tumor was submitted to the pathology department with the clinical history of neuroendocrine tumor. Gross pathology revealed about a 12 by 10 by 7 tan white heterogeneous lobulated soft mass with a central hemorrhagic scar, nearly replacing the left upper lobe. Neurologic exam revealed a triphasic tumor with primitive blastemic, mesenchymal and glandular components. Pictured here is an area of epithelial components. We can see in the higher power view that the nuclei are actually not characteristic of neuroendocrine origin. Immunohistochemistry stains were notable for Wilms tumor one and thyroglobulin negativity. Here the neuroendocrine cell markers of neuron specific enolase, synaptophysin and chromogranin were also negative. Again at this time our team was unaware that the patient's resected childhood nephroblastoma, however the triphasic morphology we have just seen is classic for nephroblastoma. Coupled with this unusual immunostain profile, our team began to suspect metastatic Wilms tumor and as such further stains were tested. Here we see the biphasic area staining strongly for TTF1, which can occur in lung and thyroid tumors. Pax8 stains were positive as well, suggesting a renal origin as well as a momentum of mesenchymal tissues. So that's odd. The presence of a positive TTF1, negative WT1 and a pulmonary metastasis of a morphologically typical nephroblastoma, this was unexpected. But after a thorough search of the literature we did conclude our diagnosis to be nephroblastoma. Wilms tumor and nephroblastoma is a pediatric malignancy that's infrequently reported in adults. While relapses typically occur within four years after the initial diagnosis, late relapse of isolated Wilms tumor in an adult is rare with few cases reported in the literature. So why is this case important? First our case is the latest relapse of Wilms tumor reported to date, occurring more than 40 years after the initial diagnosis. Also the unique immunohistochemical profile with TTF1 positivity, WT1 negativity expression could have served as a potential source of misdiagnosis. And further an accurate final diagnosis was not apparent until a more creative histopathologic workup was pursued. There are few cases reporting late pulmonary mets of nephroblastoma in adults. One such case published by Journal of Thoracic and Cardiovascular Surgery in 2006 identified a pulmonary metastasis of Wilms tumor diagnosed 17 years after the primary tumor was initially resected in adulthood. Another case published in 2005 by the Mayo Clinic describes a solitary pulmonary lesion 20 years after initial diagnosis of nephroblastoma in a two-year-old. This tumor was similarly negative for WT1 expression. The capacity for TTF1 reactivity in nephroblastoma is often unrecognized and is a potential source of misdiagnosis. This study similarly when considered with other unique features such as loss of WT1 expression, weight presenting nephroblastoma in adults or with lung metastasis. One study examined the immunophenotypic expression of TTF1, WT1, and CD56 in the primary tumor and metastatic deposits of 48 nephroblastomas. They reported TTF1 expression in one-sixth of these nephroblastomas, all of which were pediatric cases that also expressed WT1. So here's the punchline of our case. Our patient came to us with a pathology report from an outside hospital that actually outlined nephroblastoma in the differential and noted her history of Wilms tumor in childhood, but finalized the diagnosis to neuroendocrine tumor even though their immunostain profile matched ours. Unfortunately, her backstory was overlooked upon arrival to our facility. The only clinical history that was provided with the resected tumor was neuroendocrine tumor. Further still, the CT imaging revealed an absent kidney and surgical clips in the lung, so further surgical history should have been pursued and passed along. As bedside clinicians, this case should serve as a reminder to closely coordinate with our pathology and radiology departments because in order to know your future, you must know where you've been. Fortunately, our pathologists were able to keep a creative open mind and submit for secondary immunostaining. This ultimately led to the diagnosis and expands what we know of late recurring metastatic Wilms tumor of the lung. So what did we learn today? Nephroblastoma can demonstrate late recurrence in adulthood as an isolated pulmonary mass. Consider the unique immunohistochemical profiles that may still represent nephroblastoma, such as TTF1 and PAX8 positivity, as well as WT1 negativity. And take a thorough history up front and coordinate closely with pathology and radiology. Thank you to Dr. Jagadir for contribution of this case. And don't forget to evaluate the app. Hello, everybody. My name's Diana Song. I'm one of the second year fellows at Memorial Sloan Kettering in New York. And again, I'll be presenting a rare case of immunosuppression-induced pulmonary alveolar prognosis years after a hematopoietic stem cell transplant. I have no financial relationships to disclose. So our patient is a 57-year-old male with history of follicular lymphoma, status post allo-stem cell transplant, and distant history of head and neck cancer, no evidence of disease, who presented to our clinic with a dry cough in June of 2021. The cough began a few months prior after cycling and running and progressively worsened to the point that he had to stop exercising. It also worsened during and after meals and when supine. It did not keep him up at night, but getting up to use the restroom could provoke the cough. He also complained of xerostomia, requiring frequent oral rinses and pre-meal sevilamine. Two weeks prior, he was started on daily Pantoprazole due to a remote history of GERD-related cough and denied any kind of improvement. And regarding his cancer history, his follicular lymphoma was diagnosed in 2016. He received an allo-stem cell transplant in February 2018 due to relapse despite complete response in 2017. His transplant course was complicated by skin, oral, and GIGVHD. For his further history, no other significant medical history, he was born in Puerto Rico, currently resides in Connecticut. He's an educational consultant, previous tobacco chewer, social alcohol use, denied any kind of recreational drug use, and no known exposures to TB, asbestos, toxins, or animals. His vaccinations are up to date. Family history was non-contributory and he had no known drug allergies. So for his physical exam, this was limited due to a telehealth visit. He was occasionally coughing throughout the exam, but otherwise it was unremarkable. So just for a timeline, after his initial pulmonary visit in June, July 2021, we did a modified barium swallow study given his symptoms, which was negative for aspiration. CT showed diffuse patchy ground glass opacities and consolidative opacities, and he had a 0.7 centimeter right lower lobe nodule. A month later, he underwent a bronchoscopy with a right lower lobe transbronchial biopsy with cellular interstitial pneumonitis. So given the concern for pulmonary GVHD, he was started on the medications listed. From August to September of 2021, his cough improved with a prednisone taper and a CT did show improvement of his opacities. And then in September of 2021 through the spring of 2022, unfortunately he had multiple recurrences of his cough requiring prednisone and rexalitinib to be added. He also had an EGD given his recurrent GI symptoms as well that was consistent with his GI GVHD, and belumosadol was added in December of 2021. In June of 2022, he had a high resolution CT scan that showed new bilateral patchy GGOs. PFT showed possible mild restriction, infectious workup was negative, and he was started on Levaquin for possible aspiration. He was also started on extracorporeal photophoresis for his GVHD. And in July, August 2022, his cough improved despite prednisone taper with the ECP and Levaquin. And then finally in September 2022, unfortunately again, his cough worsened and did not improve despite antibiotics and prednisone increases. So his PFTs was slightly reduced DLCO of 77% and then a high resolution CT was repeated. Before we get into the imaging, just wanted to go over the labs really quick. Pretty grossly unremarkable except for his LDH was elevated at 359 and compared to his June 2021 when he first presented it was 213, so a slight elevation of that. So this is a July 2021 scan when he first presented to our pulmonary clinic. You can see the right lower lobe nodule, that was 0.74 centimeters. And he had also patchy ground glass consolidations throughout. And this was his September 2022 scan. So on this scan you can see bilateral ground glass opacities with superimposed intralobular septal thickening consistent with a crazy paving pattern. So he did undergo another bronchoscopy. We did a right lower lobe cryobiopsy which you can see the specimens here. And on a more high-powered view, so when we look closer up to our cryobiopsy specimen, you can see dilated alveolar spaces filled with granular eosinophilic proteinaceous material. The normal alveolar architecture is preserved throughout and there's no increase in interstitial inflammation or fibrosis. And you also don't see any kind of microorganisms including the AFB stains on these specimens. To the right we can see the PAS stain highlighting the granular material within the alveolar spaces. So the overall pathology was consistent with pulmonary alveolar proteinosis. Just for an extra kind of tidbit for his case, his BAL culture was positive for MAC. However the AFB stains were negative. So overall our diagnosis is a secondary PAP likely due to immunosuppressants. So as we know, PAP is a rare lung disorder caused by excessive accumulation of lipoproteins in the alveolar spaces due to dysfunction and surfactant clearance by alveolar macrophages. And the GM-CSF or granulocyte macrophage colony stimulating factor pathway is what regulates this clearance. Therefore, the disruption of GM-CSF either by autoantibodies or mutations can cause PAP. There have been rare cases of PAP reported following the use of immunosuppressants in post-stem cell transplant patients. Ruxolitinib has actually been linked to these cases of secondary PAP due to its inhibition of the Janus kinase inhibitors 1 and 2 which are crucial to the macrophage GM-CSF signaling pathway. However, there are very few cases reported of secondary PAP occurring several years after BMT and graft demand. There are very few diagnosed antemortem cases with suspected ruxolitinib due to secondary PAP more than two years after transplant in hematologic malignancy patients. Our patient is actually five years away from his transplant and he was on ruxolitinib for one year prior to the diagnosis of PAP. So regarding our patient, we completed workup with an anti-GM-CSF antibody which was negative because as we know, autoimmune PAP is the most likely cause of pulmonary alveolar proteinosis. His ECP and ruxolitinib was discontinued shortly after diagnosis and he was tapered off of prednisone by the next CT scan which was five months after the diagnosis which showed nearly resolved GGOs with waxing and waning nodular opacities. He was able to ride his bike and run once again after tapering off these medications. His MAC positive BAL culture, we consulted with ID as well and decided that we would just continue to follow up sputum cultures for him and not treat just given that his symptoms significantly improved without any kind of treatment. So overall, physicians should be aware of this complication even if patients are multiple years post engraftment, especially if on immunosuppressants because prompt removal of any offending agents can prevent mortality. Good morning, everyone. I'm Dr. Bhanu Samia, one of the second year fellows at WVU University, Pulmonary Critical Care. Today I'll be presenting about an interesting case of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia and I have no disclosures. Objectives for today would be a brief description of the case, some brief description of the epidemiology, pathogenesis, diagnosis, management and prognosis of this rare entity, DIPNIC. So going on to the case presentation, this is a 68-year-old woman who was referred to our facility from an outside center for evaluation of shortness of breath, cough and some wheezing and also an abnormal CAT scan finding. She had a past medical history that was significant for lung nodules and also some asthma, lung carcinoid that was diagnosed with biopsy and also obstructive sleep apnea for which she was treated with CPAP machine. And she also had gastric reflux. Her past surgical history, she had wedged resection of the lung times three of the right upper lobe, left lower lobe and also left upper lobe. And her social history was pretty unremarkable. She was a non-smoker and she did not have any occupational exposures. And her physical examination was grossly unremarkable at the time of our evaluation. So we did for the workup, we looked at the CAT scan images, laboratory evaluation and also some PFDs. And her laboratory analysis was unremarkable. She had no anemia. She had no eosinophilia. Her IgA levels were normal. And also her five HIAA levels were also normal. And she had a pretty normal pulmonary function testing. And her CD chest showed several nodules, less than five millimeters in size, and few nodules about six to eight millimeters in size. This is one of the image of the left upper lobe nodule and then figure B showing the right upper lobe nodule. The right upper lobe nodule had gradually increased in size from her prior CAT scans. It was about four millimeters and in like 10 years span it had grown to 1.1 to 1.5 centimeters. And one of the reasons why they referred to us was predominantly this finding of like right upper lobe, right middle lobe collapse seen in the figure A. And we did a bronchoscopy to evaluate for any endobronchial lesion given her history of lung nodules and, you know, like new cough and wheezing. And we did not see any endobronchial lesion. However, we saw erythematous mucosa all over. But otherwise it was very friable as well. No biopsies were taken at this point. Infectious workup was negative. BAL was negative for any kind of infections. And with that we did have a tumor board discussion regarding what to do regarding this patient because she's had three surgical resections for similar appearing nodules with a history of obstructive, you know, lung disease like wheezing and cough. And upon thorough review of her case and unfortunately we didn't have the pathology slides of, you know, previous like the 2006 and the 2011 scans. But we had one recent which did show carcinoid tumor. The reports did say carcinoid tumor. We didn't have the slides to evaluate. Upon tumor review board we decided like let's see if this continues to be carcinoid tumor or something else. And we did a special scan called gallium 68 labeled somatostatin receptor PET CT scan. And guess what? It did positive. And it was, so then we diagnosed her to have like, you know, neuroendocrine carcinoid tumor. With her history of a classic presentation of, you know, 10 years ago when she was 58 years of age, she had obstructive lung disease symptoms and then she had this multiple carcinoid tumors times three, had this lung resection procedure. And also the repeat PET CT scan being positive, we made an assumption of, you know, DIPNEC as a diagnosis for her. And we decided to not pursue with surgical resection of this lesion and continue to monitor surveillance. So going a little bit about DIPNEC itself, it's a rare premalignant condition. It's a newer entity. It's usually seen in female and also mean age is about 58, but mostly 50 to 60 years of age is the common presentation, often seen in nonsmokers. And it's caused due to diffuse proliferation of the neuroendocrine cells in the epithelium of the bronchial wall. And WHO defines it as a generalized proliferation of scattered single cells, small nodules or linear proliferation of pulmonary neuroendocrine cells that may be confined to the bronchial or epithelium or it can have extra luminal proliferation when they can be referred to as tumorlet when they're less than five millimeter in size or greater than five millimeter size are referred to as often carcinoid tumors. This is just an image of a carcinoid tumor. As you can see here, this is the bronchial or epithelium and just cartilage defining that this is in the bronchium. And these are the characteristic appearance of the carcinoid tumor that is organized ovoid cells. Like they tend to like stay together and then they have this stippled nucleus and also the chromatin material in there, it's called a salt and pepper appearance. The most common symptoms of the patients will often be like cough, dyspnea, exertion, wheezing. And the reason is because of this neuropeptide-induced bronchoconstriction and as well as fibroblast activation. PFT often can be cancer-obstructive parameters or it can be restrictive or it can even be normal, so not very specific. And incidental lung nodules in the CT scan is often the most common way they get further workup performed and that's why the diagnosis in itself is pretty delayed. And tissue biopsy is required for diagnosis. And this is just a picture of the carcinoid tumor showing that salt and pepper appearance and how do you differentiate that this is not a small cell carcinoma? Like this one will have a nucleus present in it versus your small cell will not have a nucleus and will have a bizarre shaped appearance. And of course you can also do special stains and special stains, this is a picture of a chromogranin, and this is for a synaptophesin, both of which are positive in these patients. And they can also have CD56 positivity. Treatment depends upon clinical symptoms. There is no clear guidelines on treatments. There are some studies which have shown steroids therapy to be helpful, inhaled versus oral. The thought is anti-inflammatory action of it. In patients who have somatostatin analog positive, we can try octreotide, but again, and other medications like inhibitor of mTOR, such as everolimus and surgical resection. Prognosis is quite variable. You can have surveillance scans, PET-CD scans, and you can have multidisciplinary team discussions so that what happened in our case of multiple resections does not happen, and you can actually follow these patients as a surveillance scans. Going back to our patient, she still continues with us in the pulmonary and also oncology services. She gets surveillance CT scans every 12 months, and she's had a stable size of the nodule. As we noticed, from four millimeter, it only went up to 1.5. It's a pretty slow-growing tumor. The rest of the tiny five millimeter, less than five millimeter nodules continue to remain stable. And her symptoms are very well controlled with inhaled corticosteroids. And one of the thought process was why not octreotide in this patient? She did not have any symptoms of carcinoid syndrome or flushing because of the benign course. The oncologist decided, you know, let's stay away from any kind of medications and follow her through. And she's doing quite okay. Good morning. Mahalo for coming this morning. The title of my case is When the Family Magic Cure Gets You Sick. I'm Dr. Javier Torres-Berrios, Pulmonary and Critical Care Research Fellow of San Juan City Hospital in Puerto Rico. I have nothing to disclose. The lesson objective of this session, I plan to share with you how a popularity, to share with you the consequences of a home remedy when used excessively. We have a 46-year-old female who comes to the ER complaining of shortness of breath, cough and weakness. She had multiple hospitalization and clinic visits being treated as pneumonia for the past two years. The patient is a former smoker and a chronic user of pentolatum-based oil for every respiratory symptoms. On physical examination, the only pertinent thing is decreased vascular breath sounds on vital signs, nothing remarkable. Lab workup, we can see a little slight increase in the ESR. The working hours are remarkable. On checked X-ray, we can see vascular opacities more prominent in the central area. These are the CT scans that we can appreciate bilateral glandular opacities along with septal thickening and air bronchograms. This is our differential diagnosis on this patient. Some of the lab workup that we send to this patient can get during the hospitalization. We start treating the patient with IV antibiotic for cat coverage, also IV solubilator based on our patient history. Due to patient multiple pneumonia, we perform a bronchoscopy, the one negative. The other negative at that time. And then on a follow-up visit, wedge-lung biopsy was performed with abundant intraalveolar fomin-leading lipid-laden macrophages seen. These findings added to patient use of pentolatum-based oil over the year led us to a diagnosis of lipoid pneumonia. That is an accumulation of endogenous or exogenous lipid in the alveoli. This magic cure is commonly used among the Hispanic for multiple symptoms, specifically of respiratory nature. The recommendation to this patient was to discontinue the exposure and to continue her asthma therapy. This X-ray on the right side is two months after being discharged home. With a slight decrease on the opacity at the right side. Thank you and don't forget to evaluate this patient. Thank you. So, very interesting case. So how much was the patient exposed to the petrolatum? Was it a chronic use, was it more of an acute use, or how much was the patient, and was it inhaled? It was a chronic use for the past two years, patient has been presenting with these symptoms, and he used it on her chest and her nostril for about two years, continuously. All right, hello everyone, a very good morning to you all. My topic of discussion for today is cannabis as an evolving cause of pulmonary Langerhans cell histiocytosis. My name is Ibrahim Zayed, and I'm a resident physician at Western Michigan University Internal Medicine. I have no financial disclosures to make. So before I begin with the topic, I'd like to ask the audience, if any of you has seen a case of pulmonary Langerhans cell histiocytosis, or come across any patient with that, I see like two nods there, okay, three. So yeah, that's what I expected, it's a pretty uncommon diagnosis. And if you guys remember that case, how many of, if those patients had a positive history of tobacco smoking, yes, and yes. So I see two yeses, and three, okay. So it's mostly a diagnosis for patients who have a history of tobacco smoking, and that's how it has been related. With Langerhans cell histiocytosis, the diagnosis is so uncommon that there's not much prevalence reported in the literature. One of the papers say that it is, the prevalence really depends on the frequency of smokers in the population, and others report like three to 5% of the total adult lung parenchymal diseases. So let's go ahead with it. I would like to thank my mentor who helped me with this case, Dr. Brandon Hooks, and these are the learning objectives for my case today. So let's take a step back and talk about cannabis a little bit. It is now a pretty widely used recreational drug in the U.S. More than 18% of the population in 2021 reported using cannabis pretty frequently. And legalization of cannabis in the U.S. has actually played a significant role. Research shows that legalization has increased the consumption and health consequences related to cannabis use. And there was one study done in 2023 which showed that 24% of the mean increase in cannabis use frequency could be actually attributed to legalization of the drug in the country. So let's talk about Langerhans cell histiocytosis a little more. So it is an infiltrative cystic lung disease, and what happens in the disease is there's a reactive proliferation of Langerhans histiocytic cells which leads to peribronchial inflammation and destruction causing diffuse multisystic interstitial lung disease. It has been classically associated with tobacco smoking like we talked about it, and it's so commonly related to tobacco smoking that it's been called a smoking-related illness. The prevalence is usually common, it's common in 20 to 40 years of age. So talking about my case, it was a 52-year-old male with the following demographics. And what was pertinent here was the history of smoking, but it was not tobacco smoking, it was actually cannabis smoking. We repeatedly asked the patient if he smoked any tobacco in his life and he denied that, but he was smoking almost like 10 blunts of cannabis every day for the past 20 years. So talking about his presentation, he had a past medical history of arthritis in his bilateral hips and wrists, but what he presented with was chest wall pain and productive cough for the past several months. He did have a history of weight loss. He had lost about like 21 pounds unintentionally in the past several months, and on physical examination all we saw was clubbing. So talking about symptoms in Langerhans cell histiocytosis, what has been reported in literature, most common symptom is actually dyspnea on the far left. And then as we come to the right side, there is nonproductive cough, fatigue, weight loss, chest pain, which our patient had, and then fever is also reported in certain cases. This was basically our diagnostic approach for our patient. We started with blood work. In his history of arthritis, he was actually following up with rheumatology as well. So he did have a positive rheumatoid factor, but the rest of the workup like ESR, CRP, quantifier on alpha-1 antitrypsin and ANA titers were all negative. So we went ahead and did a pulmonary function test, which showed signs of mild COPD, and then we resorted to radiology and biopsy. So talking about radiology, you can certainly make a diagnosis if you have a characteristic clinical presentation for Langerhans cell histiocytosis along with the findings on an HRCT. Now, CT findings would include bizarrely shaped cysts with upper lobe predominance. That's pretty classic for Langerhans cell histiocytosis. It does spare the claustrophenic angles and the ill-defined upper lobe nodules with preservation of the lung volume usually. This was the CT chest of our patient and I did not put an arrow there, but you could I think very well see the cysts which are present in the upper lobes in the two slices. I hope you guys are able to see that. So talking about further diagnosis, if you're unable to make the diagnosis on the basis of clinical presentation and CT scan, then you resort to biopsy. Now lung biopsy is actually pretty characteristic for Langerhans cell histiocytosis and it shows, like we talked about earlier, peribronchial inflammatory lesions, Langerhans-like cells, and associated destruction of the bronchiolar walls and adjacent lung parenchyma. What is more important here is the immunostaining part where the Langerhans cells actually test positive for CD1a, CD207, and S100. Now in our case, this was the pathology that was obtained. Do we have any pathologists here in the audience? So I have huge respect for all the pathologists. All I see here was like different shades of pink. So I could not really tell what it was, but I did read the report and it was negative for any S100 stain. So we actually sent this pathology for expert consultation to University of Michigan. And I could not really memorize all that what they said, but I would read that out loud. For the most part, sections of the generous transbronchial biopsy show a combination of emphysema and patchy, mild post-cellular fibrosis characteristic of so-called smoking-related interstitial fibrosis. Against this backdrop, a single piece shows fibrosis with a more stellate configuration, the central focus comprising conventional pigmented histiocytes and dense collagen fibrosis. This stellate nodule is accompanied by an equally distinctive pattern of parasympatricial airspace enlargement. And this combination of features is characteristic of the late fibrotic stage of Langerhans cell histiocytosis, a stage in which diagnostic Langerhans cells have disappeared, thus negating the diagnostic value of special stains. So this is basically why our patient's pathology didn't show any positive immunostaining. And looking back at the slide, I think I can see some stellate regions which indicate with the Langerhans cell histiocytosis. So thanks to pathology for that. Coming on to the last part, which is the treatment, it's basically smoking cessation. And that's been reported in the literature, be it tobacco or marijuana. And we can do follow-up BFTs. And optimal therapy is still pretty undetermined. But you can see if the patient responds to steroids. If not, then you can put in an effort for histiocytosis centers, which are their specialized centers. Or you can also actually enroll them in a trial of cladribine or citribine with appropriate profile access against opportunistic infection. These are a few other case reports which have been reported. One of a case of Langerhans cell histiocytosis in a marijuana smoker. And there was complete smoking cessation did lead to resolution of the patient's symptoms. But there was another report with the spontaneous remission of pulmonary Langerhans cell histiocytosis despite tobacco and cannabis use. So there is varying literature on that. But I think we need more studies on this uncommon disease to evaluate that. Thank you. Thank you so much. Hi, everyone. My name is Ibrahim. I'm a third-year MCAE fellow. I'm presenting this case with Dr. Kay from University of Cincinnati. So funny thing about this case, we had this patient who was admitted to us while we were covering at night in the ICU for a possible gap. He had a trach. He wasn't trach-devent. He was admitted. The ENT resident came to us and said, we have a patient who has a lot of gauze in his airway. And we need your help to help clear it. So we thought, OK, we'll admit him to the ICU. And we tried to clear his airway with the bronx, just prevent infection, facilitate, help the patient. But it turned out not to be gauze. So the learning objective of this case is to be familiar with this diagnosis. It's rare and unique. So I'm going to start with an introduction. So blastic bronchitis, also known as fibrinous bronchitis, it's a rare disease. It can happen both in adult and pediatric patients. It affects the whole bronchial tree from the trachea to the bronchioles. And causes could be both inflammatory and non-inflammatory. The pathophysiology is not very clear. There is multiple proposed pathophysiological mechanisms because of this condition. And I'll talk about them in a few minutes. So to go to the story of our patient, he was a guy in his late 60s, had an extensive history, ENT history. Basically had an oropharyngeal carcinoma, chemoradiation, surgery, ended up with recurrence for which he had a left neck dissection, salvage laryngectomy, and flap reconstruction. He ended up with multiple J-tube. And during his recovery, there was a notice that the output through the J-tube looked like a chile. It was non-stopping. So he ended up getting a lymphogram that showed truly a leak from his left chile duct. So he went back to the OR, underwent ligation of the chile duct, and was discharged to long-term facility with lary-tube, ET-tube. He was, chief complaint was, when he admitted to us, as I said, coughing and bringing up gas that was noticed coming from around the gas that is around his tracheostomy. With also increasing oxygen requirement. So the question was, does he have some obstruction in the distal airway because of that? Pneumonia. So we did a diagnostic bronchoscopy on him. We found a 3D material covering all the trachea and the bronchial tree. We sent it for cytology. It showed fibrinous material, aneucleate amorphous material with scanned cellularity. So the whole picture, given the duct ligation, extensive ENT history, and what we're seeing on cross-examination and this pathological finding. So we said it's most likely a plastic bronchitis, fibrinous bronchitis. And these are some of the pieces that our patient coughed and were able to get out from the bronchial tree. It's kind of interesting. So plastic bronchitis can be truly a debilitating condition, can cause airway obstruction and inflammation and infection. The pathophysiology is poorly understood. It sometimes could be related to an increase in the pressure and the mucosal of the airway related to increased venous pressure or impairment in the thoracic lymphatic duct drainage, as in our patient, due to ligation or obstruction of any kind, like malignancy. Among adult population, you can have both inflammatory and non-inflammatory condition. Some cases are reported after H1N1, pneumonia, silicosis, and as in our patient, thoracic lymphatic duct drainage obstruction due to duct stenosis or duct ligation. Management options of this condition, if you can relieve the obstruction with the bronchoscopy, get all the CAS system, mucolytic, anti-inflammatory drugs sometimes might help, and anything that can reduce the lymphatic flow with either low-fat diet or medium-chain triglyceride diet that decrease this lymphatic flow through the lymphatic system. There's also surgical option, like correction of the lymphatic drainage. Serolimus could help. Potential case reports mention that, especially with lymphatic obstruction or Kyle's leak. So as a conclusion, it's a real disease, it's good to be familiar with it. Can cause increased mortality and morbidity to the patient, recurrent admission, airway obstruction, respiratory failure. Sometimes this patient can require multiple bronchoscopy to clear the airway. So it's good to be familiar with it, because you can do things for it. So the moral of the story, if you get an ENT resident who come to you at night telling you there is gauze in the airway, just think about it. Any questions? Thank you.

patient

difficulty breathing

neuroendocrine tumor

nephroblastoma

late relapse

metastatic tumor

misdiagnosis

pulmonary metastasis